Immunodeficiency-related lymphoproliferative disorders: prospective data from the United Kingdom Children's Cancer Study Group Registry

Pinkerton, C. R., Hann, I., Weston, C. L., Mapp, T., Wotherspoon, A., Hobson, R., Kelly, D. A., Vergani, D., Hadzic, D., Rees, L., Burke, M. and Thomas, J. Alero (2002) Immunodeficiency-related lymphoproliferative disorders: prospective data from the United Kingdom Children's Cancer Study Group Registry. British Journal of Haematology, 118 2: 456-461. doi:10.1046/j.1365-2141.2002.03681.x


Author Pinkerton, C. R.
Hann, I.
Weston, C. L.
Mapp, T.
Wotherspoon, A.
Hobson, R.
Kelly, D. A.
Vergani, D.
Hadzic, D.
Rees, L.
Burke, M.
Thomas, J. Alero
Title Immunodeficiency-related lymphoproliferative disorders: prospective data from the United Kingdom Children's Cancer Study Group Registry
Journal name British Journal of Haematology   Check publisher's open access policy
ISSN 0007-1048
1365-2141
Publication date 2002-08
Sub-type Article (original research)
DOI 10.1046/j.1365-2141.2002.03681.x
Open Access Status Not yet assessed
Volume 118
Issue 2
Start page 456
End page 461
Total pages 6
Place of publication Chichester, West Sussex, United Kingdom
Publisher Wiley-Blackwell Publishing
Language eng
Abstract Clinical data and biological samples were prospectively collected in 42 children with lymphoproliferative disease (LPD) secondary to organ/bone marrow transplant-related immunosuppression (30:11 liver, 10 heart/lung, 8 kidney and 1 bone marrow), other drug-induced immunosuppression (2), congenital immunodeficiency (8) or human immunodeficiency virus (HIV)-related immune dysfunction (2). Ages ranged from 10 months to 17 years and there were 15 girls. Pathology was centrally reviewed and showed polymorphic features in 5 cases, monomorphic in 23, mixed pattern in 5 patients and 9 other types. Using the Revised European-American Classification of Lymphoid Neoplasms, 5 were B lymphoblastoid, 24 were high-grade B and 14 were other subtypes. Using the Pittsburgh classification, 9 were lymphadenopathic, 10 were systemic, 25 were lymphomatous and, with the Murphy grouping for non-Hodgkin's lymphoma (NHL), 10 were localized and 32 non-localized. Twenty-four out of 38 evaluable cases were Epstein-Barr virus positive. Thirty-five patients were evaluable for clonality; 24 were monoclonal and 11 were polyclonal. Reduced immunosuppression in solid organ transplant patients resulted in resolution of disease in 14/24, which was sustained in 11. Nineteen patients received chemotherapy, 14/18 evaluable responded, which was sustained in 8 cases. Seven out of 29 solid organ transplant and 10/13 other immune-deficient patients died. In the largest group of patients, solid organ transplants, no significant clinical or biological characteristics that predicted outcome were identified. In the transplant group close monitoring of response during reduction in immunosuppression is essential and the early use of B NHL chemotherapy may be effective.
Keyword Children
Immunosuppression
Lymphoma
Lymphoproliferation
Transplant
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
 
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